Therapy With Macrolides in Patients With Cystic Fibrosis

Abstract and Introduction

Cystic fibrosis affects 1/2500 individuals and is the most common lethal autosomal recessive disease in people of northern European descent. It is characterized by chronic infections with mucoid Pseudomonas aeruginosa and progressive deterioration of respiratory function. Much research has focused on the inflammatory component of the disease. Macrolide antibiotics are postulated to suppress inflammatory mediators and interfere with biofilm formation produced by P. aeruginosa. In vitro studies show promising results, and a limited number of human studies reported improvements in respiratory function with the drugs. Macrolide antibiotics are generally safe and well tolerated and may prove to be effective in patients with cystic fibrosis.

Many studies show that macrolides may have beneficial properties aside from their antibacterial activity. A detailed report of a single case led to the theory that the drugs may be beneficial in diffuse panbronchiolitis (DPB), and an open-label trial was conducted.[1] Since that time, much research has addressed the mechanism by which macrolides act and their application in various other disease states. Several macrolides have been studied, but azithromycin seems to have the most potent effect.[2,3,4,5,6]

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